Karl Ekbom

New dystonic syndrome associated with butyrophenone therapy.

SummaryAn acute syndrome in 3 elderly women with presenile dementia appeared as a side effect to treatment with methylperone (Buronil) and/or haloperidol (Haldol). In every case a tonic flexion of the trunk to the side was observed. Also a slight rotation of the trunk in the sagittal plane was noted. The rotation increased with walking and then the patients had a tendency to turn in a direction opposite to the path of walking. Postural and gait disturbances occurred as isolated symptoms and were different from the previously reported symptoms of acute dystonia with phenothiazine treatment. The symptoms were reversible and were improved by antiparkinson medication.The similarity in chemical structure of the butyrophenone preparations suggests a common, central mechanism of origin for the symptoms. A reversible functional disturbance in the monoamine transmission in the basal ganglia is a probable explanation.ZusammenfassungAls Nebenwirkung der Behandlung mit Methylperon (Buronil) und/oder Haloperidol (Haldol) wurde ein akutes Haltungssyndrom bei 3 älteren Frauen mit präseniler Demenz beobachtet. In jedem Falle kam es zu einer tonischen Flexion des Rumpfes zu einer Seite sowie zu einer leichten Rotation in der sagittalen Ebene. Die Rotation verstärkte sich beim Gang, wobei die Patienten die Tendenz hatten, sich in einer Richtung entgegen der Ganglinie zu drehen. Haltungs- und Gangstörungen kamen als isolierte Symptome vor und sind verschieden von den früher beobachteten und beschriebenen Symptomen der akuten Dystonie bei Phenothiazin-Behandlung. Die Symptome waren reversibel und wurden durch Anti-Parkinson-Medikamente gebessert. Die Ähnlichkeit in der chemischen Struktur der Butyrophenon-Medikamente macht einen gleichen zentralen Mechanismus in der Entstehung der Symptome wahrscheinlich. Als Erklärung kann man eine reversible Funktionsstörung in der Monoamin-Transmission der Stammganglien annehmen.

Patterns of cluster headache with a note on the relations to angina pectoris and peptic ulcer.

In a previous paper (Ekbom 1970), clinical features which distinguish cluster headache from migraine were discussed. One of these is the timing of attacks. Periodicity in the former condition was observed by Harris (1926, 1936) but clearly described by Ekbom (1947) and later by Kunkle et al. (1952), Sgmonds (1956), and others. Although most patients exhibit periodical headaches there are some cases who have in fact manifest chronic symptoms. The difference between these variants has been stressed by Ekbom (1947), Rooke e f al. (1962), Horton (1964), M c A r d k (1969), and quite recently by Earl & McArdZe (1969). During attack periods, the headachcs often occur at night, sometimes with remarkable regularity. Balla & Walton (1964) mentioned that towards the end of the periods the headaches usually become irregular and then stop completely. Hitherto no systematic analysis has been made of the occurrence of attacks during clusters. The present paper reports some patterns of cluster headachc not earlier dcscribed, with special reference to the pain at the beginning and end of the clusters. In one patient a remission of angina pectoris was observed during two successive clusters of headache.

Oxprenolol in the treatment of migraine.

Thirty migraine patients received oxprenolol (Trasicor ®), which is an adrenergic beta‐receptor blocking agent with weak beta‐stimulating intrinsic activity. The dosage was 80 mg three times a day. The treatment was given by a double blind cross‐over technique in which the effect was compared with placebo. Each compound was administered for 8 weeks with an intermediate week without medication (wash‐out). Oxprenolol had no significant effects in the prevention of migraine attacks. Our study lends further support to the assumption that differences may exist between different beta‐receptor blocking agents in their effectiveness in migraine prophylaxis.

RESTLESS LEGS SYNDROME AFTER PARTIAL GASTRECTOMY

In 1953, Nordlander gave an account of three patients with anaemia of varying origin (bleeding peptic ulcer, chronic nephritis, leukaemia) , in whom symptoms of restless legs developed concurrently with the anaemia, and disappeared when it was treated. He stated that in a further 10 cases of iron-deficiency anaemia and restless legs, the crecping sensations ceased in connexion with intravenous iron therapy, often after only a few injections. Ekbom (1960) recorded low values for serum iron in 25 per cent of 77 unselected, severe cases of restless legs. In a series of unselected cases of iron-deficiency anaemia, he found the restless legs syndrome in 24 per cent (Ekbom 1960) . A s p e n s f r o m (1964) reported symptoms of restless legs in 42 per cent of 80 patients with sideropenia. Otherwise, this relation between the restless legs syndrome and sideropenia has received little attention in the literature. Nor are restless legs mentioned in the textbooks as a feature of iron-deficiency anaemia. Anaemia and iron deficiency are known to be common sequelae of partial gastrectomy. Among the Scandinavian workers who have studied this question are Bruusgaard (1946) , W a l l e n s t e n (1954) and Krause (1962,1963) . Only a few cases of restless legs after partial gastrectomy have 1)ecn rcported previously ( E k b o m 1945,1956, Nordlander 1953, Krause 1963) . Ask-Upmark & Meurling (1955) found restless legs in 8 of 24 partially gastrectomized patients (cit. A s k U p m a r k 1959). No systematic study in a large, unselected series of such patients has, however, been made earlier.

HEREDITARY ATAXIA, PHOTOMYOCLONUS, SKELETAL DEFORMITIES AND LIPOMA

An account is given of a form of hereditary, cerebellar ataxia and photomyoclonus. Eight cases from 5 generations were affected. The disease seemed to be transmitted as an autosomal dominant trait. The age at onset usually varied between 35 to 40 years. The symptoms and signs consisted of a cerebellar ataxia, dysarthria and intention tremor. There was no nystagmus. All patients exhibited photomyoclonus and were extremely sensitive to photic stimuli. Other signs were dementia, kyphosis, pes cavus and lipoma localized in the nape of the neck, shoulders and back. Two patients had a partial syndrome with photomyoclonus and skeletal deformities. None of the patients suffered from epilepsy. In one case, histopathological examinations revealed atrophy within the cerebellar cortex, dentate nucleus and the posterior columns of the spinal cord. It is concluded that this syndrome belongs to a group of hereditary ataxias and myoclonus, and differs from myoclonic cerebellar dyssynergia (Ramsay Hunt) and also from a variety of familial myoclonus and ataxia (Gilbert et al. 1963).

HEART RATE, BLOOD PRESSURE, AND ELECTROCARDIOGRAPHIC CHANGES DURING PROVOKED ATTACKS OF CLUSTER HEADACHE

Bradycardia in attacks of cluster headache has been reported in a few, isolated cases (Dandy 1931, White & Sweet 1955, Kunkle h Anderson 1960, Ekbom 1968, Jacobson 1969). Horton has mentioned that a few patients exhibit bradycardia at the height of the pain (Horfon 1961). In most patients, however, the pulse rate was accelerated (Horton 1968, personal communication). The blood pressure showed no manifest changes in five reported cases (Horfon 1956, 1957, 1961, Jacobson 1969). The object of the present study was to investigate whether provoked attacks of cluster headache are associated with significant changes in heart rate or blood pressure. The data obtained have been related to the intensity of pain during attacks and compared with negative provocation tests. In addition, electrocardiographic changes, hitherto not described in cluster headache, were recorded in some of the patients.

Heritability of migraine as a function of definition.

The objective was to examine the main genetic and environmental architecture of migraine in the Screening Across Lifetime of Twin (SALT) Study as a function of definition. We performed a quantitative genetic study of the Swedish population and a total of 12,095 twin pairs aged 41–64 years were interviewed on the telephone by trained lay personnel using a structured questionnaire. Lifetime assessment of recurrent headache and two measures of migraine were obtained, diagnosis in line with the 1988 International Headache Society criteria and self-report. The lifetime prevalence was 10.3% for diagnosed and 21.4% for self-reported migraine. Among diagnosed cases 82.3% self-reported migraine. Among self-ascertained cases, 60.3% did not fulfil diagnostic criteria, and one third had not had recurrent headache. In multivariate analyses, genetic influences were common to diagnosed and self-reported migraine (42%) and specific to self-report migraine (11%). In univariate analyses, heritability was stable (52%) when “false positives” were removed from the case definition (diagnosed or self-reported migraine) and decreased the prevalence from 23 to 16% but dropped (from 52 to 38%) when “false negatives” were removed and decreased the prevalence from 23 to 17%. A relationship between the definition and the heritability of migraine was demonstrated and phenotypes of clinical and aetiologic relevance were disclosed.

EFFECT OF INDUCED RISE OF BLOOD PRESSURE ON PAIN IN CLUSTER HEADACHE

Attacks of cluster headache are regularly induced by the administration of nitroglycerin provided that the patient is in an active headache period and that the test is not carried out in the refractory phase following an earlier attack (Ekborn 1968a). This simple test provides a possibility of studying the basic mechanisms of the headache. Spontaneous attacks are not seldom preceded by a shift of the vegetative tone in a parasympathetic direction. Furthermore, the attacks are fairly often associated with symptoms indicative of increased parasympathetic activity, e. g. bradycardia. Two of our patients noticed that heavy physical exercise had a positive effect on spontaneous headache attacks. This suggested that an increased tone of the sympathetic nervous system might have a favourable influence on the headache. We therefore studied the effect on the pain of a rise in blood pressure induced by constant prolonged physical exercise or infusion of noradrenaline. Preliminary results of a series of exercise tests, which served as a pilot study, have been published earlier (Ekborn 1968b).

Carbamazepine, a new symptomatic treatment for the paraesthesiae associated with Lhermitte's sign.

SummaryCarbamazepine (Tegretol®) was found to have an immediate and reproducible symptomatic effect on the paraesthesiae associated with Lhermittes sign in 3 patients. The preparation was administered in a dose of 400–600 mg/day and resulted in the disappearance of the paraesthesiae within 24 hrs. When carbamazepine was withdrawn there was a recurrence of the paraesthesiae in 2 of the 3 patients. The favourable effect of the preparation was confirmed by a series of several short treatments in 2 of the patients.

Cluster Headache and Aura

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